RESUMEN
Sex cord tumor with annular tubules (SCTAT) is a rare ovarian tumor. It belongs to sex cord and stromal tumor of the ovary and represents less than 1% of cases. It includes two forms: the first one associated with Peuz-Jeghers syndrome and the second sporadic. We report 4 cases of SCTAT collected at the department of pathology of Salah Azaiez Institute of Tunis over the 12 last years. The age ranged from 10 to 32 years. Symptoms were non specific except for one case revealed by precocious puberty. One patient had Peutz-Jeghers syndrome associated. Tumors were unilateral. Gross findings showed often a solid tumor with yellow cut surface. Their size ranged from 0.5cm to 28cm. Their morphological features were characteristic. Immunohistochemistry showed that tumor cells expressed inhibin and claretinin. The treatment was surgical, often conservative. The diagnosis of malignancy wasn't focused on histological features, but on tumor extension, clinical course, and presence of metastases. Evolution was often favorable. We also performed a systematic review of the literature that identified 166 cases. Features of these cases were studied. We also compared these features between sporadic and syndromic forms and between benign and malignant forms. In conclusion, SCTAT is a rare tumor, usually benign. Its diagnosis is based on histological examination. There is a malignant potential especially in sporadic forms, estimated at 20%. Treatment is most often conservative, based on oophorectomy.
Asunto(s)
Neoplasias Ováricas , Síndrome de Peutz-Jeghers , Tumores de los Cordones Sexuales y Estroma de las Gónadas , Femenino , Humanos , Niño , Adolescente , Adulto Joven , Adulto , Tumores de los Cordones Sexuales y Estroma de las Gónadas/diagnóstico , Tumores de los Cordones Sexuales y Estroma de las Gónadas/cirugía , Neoplasias Ováricas/diagnóstico , Neoplasias Ováricas/cirugía , Neoplasias Ováricas/patología , Síndrome de Peutz-Jeghers/patología , Ovariectomía , InhibinasRESUMEN
BACKGROUND: Nephroblastoma is the most common childhood abdominal malignancy. Many studies allowed a better understanding of prognostic factors and they permitted to adapt treatment according to a risk stratification approach. AIM: To assess the most significant factors influencing the survival of patients presenting nephroblastoma. METHODS: We conducted a retrospective study over a 10-year period between 2001 and 2010 including 42 nephrectomy specimens, assessed in the pathology department of Salah Azaiz Institute, from all children diagnosed with nephroblastoma. The tumors were subdivided into histological subtypes and histological risk groups according to the SIOP-2001 classification. Statistical analyses were performed using the Kaplan-Meir and the Cox regression methods. RESULTS: The median age was 38 months. The mixed type was the most common (40% of cases). The tumors were subdivided into intermediate histological risk group (81%) and high risk group (14%). The tumors were classified as stage I (38%), stage II (24%), stage III (9%), stage IV (17%) and stage V (12%). The four-year survival rate was 83% and the event free survival rate was 85%. Age, laterality, histological risk group, tumor volume, blastema volume, stage, capsular rupture and incomplete resection had a significant impact on survival. Predictive factors of relapse were: laterality, tumor volume, blastema volume, histological risk group, stage, capsular rupture and incomplete resection. CONCLUSION: Histological type and stage were identified as the most important prognostic factors in nephroblastoma. Further large studies are needed to establish the impact of absolute blastemal volume.
Asunto(s)
Neoplasias Renales/diagnóstico , Neoplasias Renales/epidemiología , Tumor de Wilms/diagnóstico , Tumor de Wilms/epidemiología , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Niño , Preescolar , Estudios de Cohortes , Femenino , Humanos , Lactante , Neoplasias Renales/patología , Neoplasias Renales/terapia , Masculino , Recurrencia Local de Neoplasia/diagnóstico , Recurrencia Local de Neoplasia/epidemiología , Recurrencia Local de Neoplasia/terapia , Estadificación de Neoplasias , Pronóstico , Radioterapia Adyuvante , Estudios Retrospectivos , Factores de Riesgo , Tasa de Supervivencia , Túnez/epidemiología , Tumor de Wilms/patología , Tumor de Wilms/terapiaRESUMEN
Primary cutaneous plasmacytoma (PCP) is a rare cutaneous B cell lymphoma. We report a case of PCP in a 64 year old woman presenting with a nodular lesion of the left cheek. Histologically, the lesion was composed predominately of variably maturated plasma cells with monotypic expression of lambda chain. Extracutaneous localizations of the disease had been excluded. The prognosis of PCP is better than that of the metastatic cutaneous lesion of myeloma. The main prognosis factors are the size tumor and clinical presentation (solitary, versus multiple lesions). Solitary lesions of the PCP are treated by surgical excision and sometimes local radiotherapy.